Monday, November 28, 2011

Things You Don't Want - Fatal Familial Insomnia

Since it's the season of giving and receiving, one of the most talked topic of the month is "getting things". From Black Friday to "Cyber Monday", getting things for cheap is all the rage. However, while you can probably get a great deal at your local Best Buy or internet retailer that you'll love, I'll give you one thing that you probably wouldn't want, for free. Ladies and gentlemen, allow me to introduce you to, Fatal Familial Insominia, a disease so horrible that it'll practically turn your brain into a ticking time-bomb.

Pictured: Fatal Familial Insomnia?

Fatal familial insomnia or FFI, is one of the rarest diseases of the brain that you can inherit in the entire big, bad world. Only 40 families in the entire world have been recorded to have such disorder in which their DNA contains a mutated version of the gene PrPc. If you're in one of the lucky few families, it only takes one parent to give their offspring (You!) a 50% chance of taking on the gene and developing the disease later in life. Luckily, you can still get this disease spontaneously without any family history indicating the disorder. (In which case it would be called sporadic fatal insomnia, SFI, or extremely unlucky.)

But what does FFI/SFI do exactly? We've all heard of the words "insomnia" and "fatal", so it has to do something with inability to sleep and death, right? Right you are! You're on a roll today! But kidding aside, FFI is an incurable disease that involves a slow painful path to death. People that suffer from FFI typically have 18 more months to live before they finally succumb to the dementia-like symptoms of the disease and die.

Move unrelated.

The first stage of FFI lasts for about four months and hides in the shadows of a "bad night", every night. The victim will suffer from increasingly worsening insomnia, followed by panic attacks, paranoia, and extreme phobias. Sadly, the first stage only sets the stage for the last three stages before inevitable death occurs. The second stage brings the horror in horrifying, in which the victim will begin to suffer from hallucinations and the previous panic attacks will become more evident,  the victim will fall into a increasingly worsening spiral for another five months.

In the third stage, (about nine months into FFI) health deterioration would have already been obvious, other than the constant panic attacks, inability to find restful sleep, extreme paranoia, and actual hallucinations, the victim will begin to rapidly lose weight for another three months. As the insanity takes it's toll on the victim's brain and body, the final stage will settle in, dementia. The victim will quickly become unresponsive and mute for the next six months or so until death arrives to end the process.

"My constant paranoia for __________ is obviously a indicator for this disease! I must abandon all social connections, financial links, and all hope for a future immediately!" - You

Some other symptoms that are more "mild" and not as crazy as the ones listed above are, extreme sweating, sudden menopause for women and erectile dysfunction for males, neck stiffness, heighten blood pressure and heart rate, and mild constipation.

Since this disease is usually genetically transferred to another individual, it would be pretty useless if it instantly attacked the body at birth. Instead, the onset of the disease will begin around the ages 18-60, averaging at 50. Death can occur between 7 to 36 months after the first stage begins, thus giving the individual a false sense of hope for a future in the case that the person is not in the 50% window of death that will get the disease if your parent already has it. Even if detected during childhood, one could only prepare for the worst due to the fact that there is no cure or treatment for Fatal Familial Insomnia.

Gene therapy has been unsuccessful in curing the disease. Several cases have proven that sleeping pills and depressants do not help whatsoever and actually speeds up the disease's process. In one case, a man named Michael Corke was admitted into a hospital not long after his 40th birthday due to his inability to sleep and the deterioration of both his mind and body. He was "diagnosed with Multiple Sclerosis" and his doctors chose to induce a coma with sedatives; they found that his brain continued to remain active even after coma-inducing drugs were administered. He died six months later after his sleeplessness began.

So when you go to sleep tonight and find yourself rolling around wondering if you could be one of the 100 individuals in the world that have this disease, worry not, you probably don't have it. (And if you do, you still can't do anything about it. Blissful ignorance they call it.)

Pictured: You.